ÖZET
Klinigimize, 6 senedir her iki gözde görme azligi yakinmasi ile basvuran 58 yasindaki erkek hastaya ayrintili göz muayenesi yapildi ve fundus floresein anjiografisi (FFA) çekildi. Hastanin görme keskinligi; sag göz 50 cm, sol göz 30 cm'den parmak sayma düzeyindeydi. Göz dibi muayenesinde bilateral makula bölgesinde hiperpigmente genis skar ve optik disk nazalinde anjiyoid striyalari (AS) düsündüren lezyonlar izlendi. Hastanin FFA'sinde bilateral peripapiller AS'lar tespit edildi. Olgunun makulalarindaki hiperpigmente skarlarin AS'lara ikincil gelisen koroid neovaskülarizasyonlara bagli oldugu düsünüldü. Sistemik muayenesinde, psödoksantoma elastikumdaki cilt lezyonlarina benzer bulgularin görülmesi üzerine hastaya Dermatoloji konsültasyonu istendi. Dermatoloji bölümünde yapilan muayene sonucu psödoksantoma elastikum tanisi kondu. Bu bulgularla hastanin Groenblad-Strandberg sendromu (psödoksantoma elastikum ve anjioid striya birlikteligi) olduguna karar verildi. Fundusta anjioid striyalarin görülmesi halinde akla ilk gelmesi gereken hastalik psödoksantoma elastikum olmalidir. Ancak, peripapiller yogun skar ve atrofi olmasi halinde anjioid striyalar net görülemeyebilecegi için FFA ile taniya ulasilabilir.
Anahtar Kelimeler: Anjiyoid striya, Psödoksantoma elastikum, Groenblad-Strandberg sendromu
Groenblad-Strandberg Syndrome: A Case Report
SUMMARY
A fifty-eight-year old male patient with a history of visual loss for 6 years underwent a detailed ophthalmological examination including fundus fluorescein angiography (FFA). Visual acuity was at the level of counting fingers (50 cm OD and 30 cm OS). Ophthalmoscopically, hyperpigmented macular scars and peripapillary lesions similar to angioid streaks (AS) were observed bilaterally. FFA revealed bilateral peripapillary AS. Hyperpigmented macular scars were thought to originate from choroidal neovascularizations secondary to AS. During his systemic evaluation, skin lesions similar to those of pseudoxanthoma elasticum (PXE) were observed, and a dermatology consultation was obtained. Patient was diagnosed as PXE in Dermatology department. Hence, it was decided that patient was a case of Groenblad-Strandberg syndrome (combination of AS and PXE). PXE should be the first disease to remember when AS were observed in fundus. However, if are not readily visible due to peripapillary scar tissue and atrophy, FFA is a valuable tool in diagnosis.
Key Words: Angioid streaks, Pseudoxanthoma elasticum, Groenblad-Strandberg syndrome
